Clinical Care

Early referral to a paediatrician with special responsibility for epilepsy (within 2 weeks of first seizure)
Development of a comprehensive care plan
Regular review
Referral to tertiary services if there is diagnostic uncertainty or treatment failure

The services should be child-centred, and the review should provide access to written and visual information about their condition, counselling services, voluntary organisations, epilepsy nurse specialists and integration with other community and multi-agency services involved in children’s education, welfare and well being. This integration may commonly be mediated by the epilepsy nurse specialists.

Many of these recommendations are very appropriate in LKS, once the diagnosis has been made. However, there are difficulties as these guidelines refer to the case of clinically apparent seizures, which do not always occur in LKS (and in any case, are not the main problem). Furthermore, there is often a significant time delay before the diagnosis of LKS is made, so care pathway recommendations must be slightly modified, as below.

Early referral to a paediatrician should be triggered either by a seizure OR loss of language abilities without overt seizures. In LKS, children demonstrate loss of previously acquired language abilities in association with subclinical seizure activity, although this may initially be mistaken for other conditions (e.g. mutism, deafness, behavioural problems).
A paediatrician should conduct an initial assessment and investigation. Ideally, this should be a multidisciplinary assessment by the local team, including speech and language assessment, and assessment of cognitive abilities / developmental level. A paediatric neurologist would usually be involved in further assessment of such a regression, and would arrange specialist investigations (e.g. sleep EEG or video telemetry) and a multidisciplinary assessment as necessary.
After diagnosis of LKS, a paediatric neurologist would generally oversee the child’s medical management and liaise with the local paediatrician, who would be responsible for coordinating therapy and support for the child and their family.
Regular review during the active phase of the disease would involve close liaison between paediatric neurologist and paediatrician, and the facility for language and cognitive assessments (particularly to monitor response to changes in medication). There should be access to advice on appropriate educational placement, and behaviour management if necessary (child psychiatry / psychology).
The child may be referred on to a specialist paediatric epilepsy centre (such as the Developmental Epilepsy Clinic at GOSH) if there is:
a. poor response to treatment
b. further loss of skills or ‘plateauing’ in development
c. complex or severe behaviour problems
d. the possibility of epilepsy surgery